Hypogammaglobulinemia

Description:

Hypogammaglobulinemia - the state caused by the low level of immunoglobulins in blood serum.
The disease was for the first time described in 1956. It is characterized by a hypogammaglobulinemia  owing to disturbance of formation of IgG at children of early age and diagnosed after disappearance of parent transplacental IgG. The Tranzitorny hypogammaglobulinemia remains at children from 6 months to 2-3 years. The IgG level at the same time is reduced twice in comparison with age norm at normal or reduced indicators of IgA and IgM.

Children at the age of 4-7 months have a physiological hypogammaglobulinemia when the level of parent IgG decreases, and the speed of synthesis of own immunoglobulins is still insufficient and reaches norm only by 2-3 years. Earlier for the characteristic of this state used the term "slow immunological start".

It should be noted that the tranzitorny gipogammaglobulipemiya is revealed more often at children in whose families there were cases of immunodeficiencies. There is this type of an immunodeficiency at boys and girls with an identical frequency.

Gipogammaglobulinemiya's symptoms:

At some babies with a tranzitorny hypogammaglobulinemia symptoms are not shown. They normally answer antigens of vaccines and several years later "outgrow" a hypogammaglobulinemia. At other children reveal the recuring bacterial infectious diseases since first month of life. On average at 50% of such children the diagnosis is established by 6-12 months of life.

The main clinical manifestations — bacterial upper respiratory tract infections (for example, average otitis, sinusitis). Pneumonia, meningitis, sepsis develop seldom. At some children reveal recurrent diarrhea, severe forms of chicken pox, it is long the remaining oral candidiasis. At most of children allergic diseases, including bronchial asthma, atopic dermatitis, food allergy develop. Lymph nodes and almonds at such children of a gipoplazirovana. In general children normally grow and develop.

Gipogammaglobulinemiya's reasons:

The genetic basis of a tranzitorny hypogammaglobulinemia is still unknown. Assume that deficit T-helperov or disturbance of balance of cytokines takes place.

Gipogammaglobulinemiya's treatment:

ри to a symptomatic form apply fortifying and antimicrobic therapy according to sensitivity of the activator. If patients have recurrent bacterial infections, then as preventive therapy it is necessary to apply antibiotics, especially during a season when respiratory infectious diseases become frequent.

To patients at whom respiratory infections arise often and proceed hard or refrakterno, in addition to an antibioticotherapia, appoint intravenous immunoglobulins on 400-500 mg/kg each 3-4 weeks during the season. Replacement therapy needs to be begun 3-6 months later after disturbances of a humoral immune response begin to be shown clinically.

It is shown that the short course replaceable therapy by immunoglobulins does not suppress formation of specific antibodies. At most of children immunity is recovered on the 2nd year of life. As the tranzitorny hypogammaglobulinemia comes to an end more often with spontaneous recovery, pathogenetic immunocorrection is not required. Treatment of infectious diseases is carried out according to standards corresponding to a nosology. In general the forecast of a tranzitorny hypogammaglobulinemia is favorable.